AAN.com
Quick search in Citations
AnywhereCitation
Advanced Search
Trending Terms:
Articles
January 15, 2007

Outcome of noninvasive ventilation in children with neuromuscular disease

H. K. Young, FRACP, MRCP, A. Lowe, BSc, D. A. Fitzgerald, PhD, FRACP, C. Seton, FRACP, K. A. Waters, FRACP, E. Kenny, RN, L. S. Hynan, PhD, S. T. Iannaccone, MD, FAAN, K. N. North, MD, FRACP, and M. M. Ryan, MMed, FRACPAuthors Info & Affiliations
January 16, 2007 issue
68 (3) 198-201
https://doi.org/10.1212/01.wnl.0000251299.54608.13
Letters to the Editor
Track CitationsAdd to favorites
Get Access

Abstract

Objective: To assess the effect of institution of noninvasive ventilation (NIV) on clinical outcome and quality of life (QOL) in a cohort of children with severe neuromuscular disorders.
Methods: We reviewed records and obtained clinical data from the year prior to commencing NIV and annually thereafter. Data obtained included diagnosis, patient symptoms, mortality, NIV adverse effects, pulmonary function tests, polysomnographic data, length of hospitalizations, and health care costs. Patients and parents completed questionnaires assessing QOL with NIV and recalling QOL before NIV.
Results: Fourteen of 17 (82%) suitable patients were enrolled. Follow-up ranged from 6 to 84 months (median 30). Symptoms of daytime sleepiness (p = 0.003) and headache (p = 0.046) improved after initiation of NIV. Sleep quality assessed by polysomnography also improved. Hospitalization rates (p = 0.002) and health care costs (p = 0.003) decreased. QOL remained stable after NIV, despite disease progression.
Conclusion: Treatment of respiratory failure, in children with neuromuscular disease, with noninvasive ventilation results in a reduction in symptoms, hospitalizations, and health care costs without adverse effects on quality of life.

Get full access to this article

View all available purchase options and get full access to this article.

Get Access

Supplementary Material

File (e1.doc)
File (young.pdf)

References

1.
Annane D, Chevrolet JC, Chevret S, et al. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev 2000;2:CD001941.
PubMed
Google Scholar
2.
Simonds AK, Ward S, Heather S, et al. Outcome of paediatric domiciliary mask ventilation in neuromuscular and skeletal disease. Eur Respir J 2000;16:476–481.
Crossref
PubMed
Google Scholar
3.
Katz S, Selvadurai H, Keilty K, et al. Outcome of non-invasive positive pressure ventilation in paediatric neuromuscular disease. Arch Dis Child 2004;89:121–124.
Crossref
PubMed
Google Scholar
4.
Wallgren-Pettersson C, Bushby K, Mellies U, et al. ENMC. 117th ENMC workshop: ventilatory support in congenital neuromuscular disorders—congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II) 4–6 April 2003, Naarden, The Netherlands. Neuromuscul Disord 2004;14:56–69.
Crossref
PubMed
Google Scholar
5.
Rechtschaffen A, Kales AD. A manual of standardized terminology, techniques and scoring system for sleep stages of human subjects. US Department of Health, Education, and Welfare Public Health Service 1968.
Google Scholar
6.
Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care 2001;39:800–812.
Crossref
PubMed
Google Scholar
7.
Iannaccone ST, Hynan LS. American Spinal Muscular Atrophy Randomized Trials (AmSMART) Group. Reliability of 4 outcome measures in pediatric spinal muscular atrophy. Arch Neurol 2003;60:1130–1136.
Crossref
PubMed
Google Scholar
8.
Raphael JC, Chevret S, Chastang C, et al. Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy. Lancet 1994;343:1600–1604.
Crossref
PubMed
Google Scholar
9.
Norregaard O. Noninvasive ventilation in children. Eur Respir J 1920;5:1332–1342.
Google Scholar
10.
Miller JR, Colbert AP, Schock NC. Ventilator use in progressive neuromuscular disease: impact on patients and their families. Dev Med Child Neurol 1988;30:200–207.
Crossref
PubMed
Google Scholar
11.
Mellies U, Ragette R, Schwake C, et al. Daytime predictors of sleep disordered breathing in children and adolescents with neuromuscular disorders. Neuromuscul Disord 2003;13:123–8.
Crossref
PubMed
Google Scholar
12.
Aldrich TK. Nutritional factors in the pathogenesis and therapy of respiratory insufficiency in neuromuscular diseases. Monaldi Arch Chest Dis 1993;48:327–330.
PubMed
Google Scholar
13.
Sritippayawan S, Kun SS, Keens TG, et al. Initiation of home mechanical ventilation in children with neuromuscular diseases. J Pediatr 2003;142:481–485.
Crossref
PubMed
Google Scholar
14.
Oostveen E, MacLeod D, Lorino H, et al. The forced oscillation technique in clinical practice: methodology, recommendations and future developments. Eur Respir J 2003;22:1026–1041.
Crossref
PubMed
Google Scholar
15.
American Thoracic Society. Standards and indications for cardiopulmonary sleep studies in children. Am J Respir Crit Care Med 1996;153:866–878.
Crossref
PubMed
Google Scholar
16.
Mier-Jedrzejowicz A, Brophy C, Green M. Respiratory muscle weakness during upper respiratory tract infections. Am Rev Respir Dis 1988;138:5–7.
Crossref
PubMed
Google Scholar

Information & Authors

Information

Published In

Neurology®
Volume 68 • Number 3 • January 16, 2007
Pages: 198-201
PubMed: 17224573

Copyright

© 2007.

Publication History

Published online: January 15, 2007
Published in print: January 16, 2007

Permissions

Request permissions for this article.

Authors

Affiliations & Disclosures

H. K. Young, FRACP, MRCP
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
A. Lowe, BSc
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
D. A. Fitzgerald, PhD, FRACP
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
C. Seton, FRACP
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
K. A. Waters, FRACP
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
E. Kenny, RN
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
L. S. Hynan, PhD
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
S. T. Iannaccone, MD, FAAN
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
K. N. North, MD, FRACP
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author
M. M. Ryan, MMed, FRACP
From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.
View all articles by this author

Notes

Address correspondence and reprint requests to Dr. Monique M. Ryan, Neurosciences Department, Royal Children's Hospital, Flemington Rd, Parkville Victoria 3052, Australia; e-mail: [email protected]

Metrics & Citations

Metrics

Citation information is sourced from Crossref Cited-by service.

Citations

Download Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.

Cited By
    • María Ángeles García-Teresa,
    • Marti Pons Odena,
    Noninvasive MV of PMVed Children, Prolonged and Long-Term Mechanical Ventilation in Children, (51-78), (2024).https://doi.org/10.1007/978-981-97-8903-0_5
    Crossref
    • Takahiro Kido,
    • Atsushi Kawaguchi,
    History and Epidemiology of PMVed Children (Including Home-Ventilated Children), Prolonged and Long-Term Mechanical Ventilation in Children, (11-20), (2024).https://doi.org/10.1007/978-981-97-8903-0_2
    Crossref
    • Marcella Aversa,
    • Susanna Staccioli,
    Adaptation-Intolerance, Delirium in Agitated Patients. Neuromuscular Disorders: Breathing and Swallowing, Pharmacology in Noninvasive Ventilation, (315-322), (2024).https://doi.org/10.1007/978-3-031-44626-9_33
    Crossref
    • Julian Legg,
    • Jamie-Leigh Allen,
    • Morag Andrew,
    • Charlotte Annesley,
    • Michelle Chatwin,
    • Hannah Crawford,
    • Joanna Elverson,
    • Julian Forton,
    • Kate Oulton,
    • Kate Renton,
    • Alison Tavare,
    • Hilary Tedd,
    • A John Simpson,
    BTS Clinical Statement on the prevention and management of community-acquired pneumonia in people with learning disability, Thorax, 78, Suppl 1, (s1.1-s31), (2023).https://doi.org/10.1136/thorax-2022-219698
    Crossref
    • Lucia Ronco,
    • Sonia Khirani,
    • Meryl Vedrenne-Cloquet,
    • Remi Barrois,
    • Christine Barnerias,
    • Isabelle Desguerre,
    • Elisabetta Bignamini,
    • Brigitte Fauroux,
    Limitations of the apnea-hypopnea index in children and young adults with neuromuscular disorders, Neuromuscular Disorders, 33, 6, (468-473), (2023).https://doi.org/10.1016/j.nmd.2023.05.001
    Crossref
    • Jason Ackrivo,
    • Hemant Sawnani,
    Chronic mechanical ventilation, Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease, (125-148), (2023).https://doi.org/10.1016/B978-0-323-95747-2.00004-5
    Crossref
    • Ángela Andrea Pesántez-Abril,
    • Adriana del Carmen Alva-Chaire,
    • Francisco Javier Cuevas-Schacht,
    Hipoventilación alveolar crónica en pacientes pediátricos a altitud moderada, NCT Neumología y Cirugía de Tórax, 81, 2, (94-102), (2022).https://doi.org/10.35366/108495
    Crossref
    • Elaine Chan,
    • Martin Samuels,
    Long Term Ventilation in Children, Encyclopedia of Respiratory Medicine, (330-362), (2022).https://doi.org/10.1016/B978-0-08-102723-3.00245-6
    Crossref
    • Kate Ching-Ching Chan,
    Sleep and Neuromuscular Diseases: Management and Use of NIV, Paediatric Sleep Disorders, (177-191), (2022).https://doi.org/10.1007/978-981-19-5791-8_19
    Crossref
    • Shay Ehrlich,
    • Inbal Golan Tripto,
    • Moran Lavie,
    • Michal Cahal,
    • Tommy Shonfeld,
    • Dario Prais,
    • Hagit Levine,
    • Meir Mei‐Zahav,
    • Ophir Bar‐On,
    • Yulia Gendler,
    • Jonatan Zalcman,
    • Eahab Sarsur,
    • Micha Aviram,
    • Aviv Goldbart,
    • Patrick Stafler,
    High flow nasal cannula therapy in the pediatric home setting, Pediatric Pulmonology, 58, 3, (941-948), (2022).https://doi.org/10.1002/ppul.26282
    Crossref
  11. See more

View Options

Login options

Check if you have access through your login credentials or your institution to get full access on this article.

Personal login Institutional Login
Purchase Options

The neurology.org payment platform is currently offline. Our technical team is working as quickly as possible to restore service.

If you need immediate support or to place an order, please call or email customer service:

  • 1-800-638-3030 for U.S. customers - 8:30 - 7 pm ET (M-F)
  • 1-301-223-2300 for customers outside the U.S. - 8:30 - 7 pm ET (M-F)
  • [email protected]

We appreciate your patience during this time and apologize for any inconvenience.

View options

PDF and All Supplements

Download PDF and Supplementary Material

Full Text

View Full Text

Full Text HTML

View Full Text HTML

Figures

Tables

Media

Share

Share

Share article link

Share

View full text|Download PDF